To preserve patient privacy and for didactic purposes, case descriptions and pathology reports have been anonymized and partially fictionalized. The pathology images are representative images from a mixture of similar cases.

Invited Speakers

John Danziger
John DanzigerCase Presenter | Beth Israel Deaconess Medical Center, Boston, MA, USA | No conflict of interest reported
 Isaac Stillman
Isaac StillmanNephropathology Discussant | Beth Israel Deaconess Medical Center, Boston, MA, USA | No conflict of interest reported
Eduardo Haddad
Eduardo HaddadNephrology Discussant | Lawrence General Hospital,Boston, MA, USA | No conflict of interest reported

Case

Patient is a 55-year-old female with thrombocytopenia and a positive ANA, who was diagnosed to have undifferentiated connective tissue disease (+anti Ro Ab, prolonged PTT) without any systemic manifestations of systemic lupus.

Her serum creatinine had been around 1.1 to 1.4 mg/dl and urinary protein around 0.1 to 0.3 g/g. More recently, a sudden rise in creatinine to 1.8 mg/dl and a newly positive dsDNA prompted referral for a renal evaluation.  

Medical history:
She has had a thrombocytopenia and prolonged PTT for many years. As part of the workup, she was found to have a lupus anticoagulant. No history of thrombosis.
Moderate-severe mitral regurgitation
Hypertension
Obesity
Obstructive sleep apnea
Hypothyroidism: Status post thyroidectomy for goiter
Urticaria
Anemia

Medications:
Hydroxychloroquinine
Warfarin
Aspirin
Levothyroxine
Metoprolol
Ranitidine
Spirolactone
HCTZ
Amlodipine

Physical Exam:
BP 146/86
BMI 28
No rash, or oral ulcers. No joint effusions. 1+ lower extremity edema

Laboratory and other data:
Creatinine 1.8mg/dl
C3 85 mg/dl, (90-180 mg/dl)
C4 14 mg/dl (10-40 mg/dl)
PTT 108 sec (25-36 sec)
Positive lupus anticoagulant
Negative anticardiolipin IgG and IgM
Negative beta-2 glycoprotein IgG
ANA 1:80
dsDNA 1:40
ESR 80 cm
CRP 10 mg/l
Positive Ro >8.0.
Positive SS-A,
Negative anti-RNP
Negative anti-Smith
Negative cryoglobulins
Hb 10 g/dl
Platelets 130 k/microliter
Urinary p/c: 0.3  to now 0.9 g/g

Kidney Pathology

Pathology images pending

1. Lupus nephritis, ISN RPN Classification Class V Membranous Lupus Nephritis.

2. Vascular changes consistent with chronic thrombotic microangiopathy, in the setting of lupus anticoagulant positivity.

Questions posed & summary of key discussion points

1. Would you treat patient’s renal findings (class V LN) in light of lack of much proteinuria?
2. How would you manage the presence of TMA and lupus anti-coagulants?

Author(s) of case summary:

Case summary pending

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